McCune-Albright syndrome (MAS), a rare multisystem disorder caused by GNAS1 gene mutation is characterized by polyostotic fibrous dysplasia of bone (FD), endocrine disorders and caf?-au-lait skin hyperpigmentation. FD affects craniofacial bones including the jaws in 90% of cases. FD/MAS patients are also associated with dental disorders that include tooth rotation, tooth displacement, missing teeth, enamel hypoplasia, enamel hypomineralization, abnormally large pulp chamber, retained deciduous teeth, attrition and severe malocclusion Dental management of FD/MAS is medically compromised by endocrine disorders and handicapping bone pain. Furthermore, bisphosphonates often used to control FD and associated bone pain poses a risk for jaw bone necrosis. Earlier subjective reports indicate dental surgery could exacerbate jaw FD lesions but there is still limited qualitative data on dental outcomes and effectiveness of dental treatments i maxillo-mandibular FD/MAS patients. Our collaborative group has access to the largest well-described and well-characterized cohort of 140 FD/MAS patients enrolled in an ongoing NIDCR/NIH natural history study of MAS. This patient population represents an appropriate cohort for intramural-extramural collaborative research that will assess outcomes of dental treatment in FD/MAS. Our objective is to determine the outcomes of dental therapies on maxillo-mandibular FD lesions in MAS patients and also the impact of FD on the prognosis of dental therapies. Using a combination of retrospective and prospective longitudinal case-cohort analysis we will assess in Aim 1 the outcomes and effectiveness of endodontic and orthodontic therapies in FD/MAS patients with maxillo-mandibular FD.
In Aim 2, we will determine whether or not dental surgery within FD is associated with delayed healing, bone necrosis and exacerbation of FD disease burden. New knowledge gained from this unique patient population with a rare disease will provide evidence-based data to support 'best clinical practice'guidelines for dental care that will improve quality of life of FD/MAS patients.

Public Health Relevance

McCune-Albright syndrome is a multisystem disorder of bone, endocrine tissues and skin. The associated bone disease called fibrous dysplasia affects different bones including the jaw bone. We will evaluate the outcomes of different dental treatments in this disorder and determine whether the jaw disease is disturbed by dental treatment.

Agency
National Institute of Health (NIH)
Institute
National Institute of Dental & Craniofacial Research (NIDCR)
Type
High Priority, Short Term Project Award (R56)
Project #
1R56DE022932-01A1
Application #
8705613
Study Section
Special Emphasis Panel (ZDE1-MH (06))
Program Officer
Atkinson, Jane C
Project Start
2013-09-25
Project End
2014-07-31
Budget Start
2013-09-25
Budget End
2014-07-31
Support Year
1
Fiscal Year
2013
Total Cost
$200,000
Indirect Cost
$75,000
Name
University of Pennsylvania
Department
Dentistry
Type
Schools of Dentistry
DUNS #
042250712
City
Philadelphia
State
PA
Country
United States
Zip Code
19104
Akintoye, Sunday O; Greenberg, Martin S (2014) Recurrent aphthous stomatitis. Dent Clin North Am 58:281-97